Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000470635 | SCV000549125 | pathogenic | Danon disease | 2018-11-13 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. While this particular variant has not been reported in the literature, loss-of-function variants in LAMP2 are known to be pathogenic (PMID: 21415759). This sequence change deletes 3 nucleotides and inserts 2 from exon 4 of the LAMP2 mRNA (c.546_548delinsTA), causing a frameshift at codon 183 This creates a premature translational stop signal (p.Ser183Thrfs*59) and is expected to result in an absent or disrupted protein product. |